The extreme burden of Hypertrophic Cardiomyopathy (HCM) prompted us to set a comprehensive HCM unit : Dr Hisham Ahmed

The Hypertrophic Cardiomyopathy unit is essentially designed on the basis of multiple spheres of care. We have fused the arms of genetics, cardiac imaging, interventional cardiology, cardiac electrophysiology, heart failure team as well as cardiac surgery to create a comprehensive HCM unit.ETHealthWorld | August 28, 2017, 08:52 IST

Why have you focused on HCM?Hypertrophic Cardiomyopathy, also known as HCM, is the commonest inherited heart diseases in clinical practise in Cardiology. We are looking at a prevalence of Hypertrophic Cardiomyopathy to the tune of one in every 500 live births. So you can imagine the extreme burden of HCM disease in the general population. We believe that Hypertrophic Cardiomyopathy patients have unique medical needs and we need a comprehensive Hypertrophic Cardiomyopathy unit to to address the special health needs of these patients.

How have you set up the HCM unit at Amrita?The Hypertrophic Cardiomyopathy unit is essentially designed on the basis of multiple spheres of care. So, in the department of Cardiology here at the Amrita Institute of Medical Sciences we have incorporated a fusion of various subsections of Cardiology to create an effective HCM unit. Essentially, we have fused the arms of genetics, cardiac imaging, interventional cardiology, cardiac electrophysiology and heart failure team as well as the very important role of cardiac surgery to create a comprehensive Hypertrophic Cardiomyopathy unit.

We were very fortunate to collaborate with Dr Barry Maron from Minneapolis who is considered the foremost authority on Hypertrophic Cardiomyopathy in the world and we have a daily working relationship with Dr Maron in terms of scientific collaboration as well as share registry data.

How do you diagnose the genomics part of it?Hypertrophic Cardiomyopathy, as I have mentioned, is the commonest inherited cardiac disease. So when the term genomics came into vogue, a couple of decades ago, every genetic test was considered very expensive to the tune of 1 to 1.5 lakh rupees. We used to analyse the genomics or genetics of a patient. Now with advances in the genomic technology we are now able to ascertain the genetic pattern or genetic mutation of a patient with Hypertrophic Cardiomyopathy with a mere blood sample to the tune of 3 to 4 ml per patient. We submit these blood samples to our genetic laboratory partners, we work with a couple of labs in the field of genomics.

We work with a group called MedGenome labs from Bangalore and work out of Bangalore and they are also based in the US in San Francisco. We have had a close working relationship with them and the turnaround time after a patient's blood sample is submitted is close to 6 to 8 weeks and they will give us information where the particular genetic mutation is identified in the patient with Hypertrophic Cardiomyopathy.

What are the challenges that you find?To begin with, one of the biggest challenges that we face in the sense was that coronary artery disease is the most prevalent form of heart disease in the subcontinent. Essentially, a majority of our work is focused on coronary artery disease. But there is an equally important segment called Cardiomyopathy of which Hypertrophic Cardiomyopathy is a subset. So the challenges that we face, as of now, is to probably divert resources to care for patients with Hypertrophic Cardiomyopathy. The recognition of Hypertrophic Cardiomyopathy, in general, in the population is so prevalent that it is important to form a comprehensive Hypertrophic Cardiomyopathy unit to meet the needs of all the Hypertrophic Cardiomyopathy patients. So these are the challenges that we faced. To overcome these challenges we have taken certain steps, as I mentioned before, to fuse the various arms of cardiology in our department to address these needs.

How do you intend to carry this forward?As of now, our HCM unit is a culmination of various spheres of cardiac subspecialties. Our objective, in the future, is to maintaining a comprehensive database and registry of all our patients with Hypertrophic Cardiomyopathy. We intend to have a prospective, a long term follow up of all these patients and you have to remember when you treat a patient with Hypertrophic Cardiomyopathy you are not only treating the patient, you are treating the entire family, as well for the simple reason that if the mother or father has Hypertrophic Cardiomyopathy then there is a 50% chance that the child has Hypertrophic Cardiomyopathy. Therefore, it is not patient focused, it is the entire focus on the family. So we intend to follow up on our patients and progress forward with family screening strategies for all of our patients and we intend to work with more efficiency in terms of bringing closer and bringing together Cardiology in order to carry forward our Hypertrophic Cardiomyopathy unit.

What is the screening strategy set in place?Essentially, what we do is when we have a patient come to us with Hypertrophic Cardiomyopathy our first objective is to gather as much data about the patient who has come to us. We do a clinical examination, we perform investigations of a patient with HCM. The second stage is equally important as we would like to screen all the first degree relatives of the patient who has come to us with Hypertrophic Cardiomyopathy. For example, if a patient has come to us we would screen his siblings, we would screen his children, and we would also screen his parents. All of them are first degree relatives. We would screen using basic investigations such as ECG and echocardiogram. Recently, with the advent of genomics and we have a comprehensive genomics arm in the HCM unit. So with the advent of genomics we do use genetic testing, gene sequencing to identify the mutation in the patient as well as the first degree relatives who are being screened.

Can you please comment on the use of gene therapy in the future?Since this is a genetic disease the age old question is whether we can offer any form of therapy in the future that can border on something like gene therapy. As of now, Hypertrophic Cardiomyopathy is a condition where there is abnormal wall thickness of the walls of the heart. So, we do not have any cutting edge genetic therapy per se to reduce the thickness. We have some surgical techniques when the Hypertrophic Cardiomyopathy becomes excessive. But in terms of real futuristic therapy like gene therapy certain experimental models are in the progress. But as of now in the future, it may be possible but at this moment we do not have any validated treatment option.

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